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Ahmad Bader, Manraj Heran, Christopher Dunham and Paul Steinbok


Two of the more common infantile brain tumors, glioblastoma multiforme (GBM) and desmoplastic infantile tumors (DITs), can be difficult to distinguish on MRI. Both tumors occur in the supratentorial compartment and both have solid and cystic components. Differentiating between the 2 on MRI studies could assist the surgeon in discussions with family and child management. The authors report on their institutional experience with both tumors, focusing on radio-graphic features, especially the diffusion studies, which might be useful in distinguishing between infantile GBM and DIT.


A retrospective review was undertaken of all infantile brain tumors treated at British Columbia’s Children’s Hospital between 1982 and 2012, and cases of GBM and DIT were recorded. Only cases that had imaging were included in the study. A literature review was completed to identify reported cases of infantile GBM and DIT. Only reports that described or included radiological studies (particularly MRI) of the tumors were included. Certain radiographic features of the tumors were reviewed, including location, size, consistency, pattern of enhancement, and features on MR diffusion studies.


Of 70 cases of infantile brain tumors, 2 GBM cases and 3 DIT cases (all 3 of which were desmoplastic infantile gangliogliomas [DIGs]) met the inclusion criteria. The radiological studies obtained in all 5 cases were reviewed by a neuroradiologist. All 5 patients had supratentorial tumors with cystic-solid consistency. Diffusion MRI studies showed restricted diffusion in the 2 GBM cases, but no evidence of restricted diffusion in the DIG tumors. The GBM tumors were heterogeneously enhancing, and the DIG tumors showed avid and homogeneous enhancement. The literature review revealed 29 cases of infantile GBM and 32 cases of DIG/DIT that met the inclusion criteria. The tumors were large in both groups. The tumors were cystic-solid in consistency in 10 of 30 (33%) of GBM cases and 28 of 32 (87.5%) of DIT cases. The contrast enhancement was heterogeneous in 9 of 30 (30%) GBM cases, and it was homogeneous and avid in 27 of 32 (84%) of DIT cases. Diffusion studies were recorded in 2 published infantile GBM cases, and in both of them diffusion was restricted. The authors only found 1 report that discussed DIG tumor features on MR diffusion studies, but the interpretation was difficult and unclear.


Magnetic resonance imaging, especially diffusion-weighted imaging, may be a useful aid in distinguishing between infantile GBM and DIT tumors, with infantile GBM demonstrating restricted diffusion.

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Navraj S. Heran, Raymund L. Yong, Manraj S. Heran, Steven Yip and David Fairholm

✓ This 42-year-old man presented with an intradural extramedullary mass in his lower cervical spine. On imaging studies the lesion mimicked the appearance of a meningioma. At surgery, the mass was found to be an intradural extra-arachnoid tumor. An intraoperative pathological examination determined the lesion to be a Hodgkin lymphoma. The procedure was limited to biopsy sampling and the patient was treated further with adriamycin, bleomycin, vincristine, and dacarbazine chemotherapy, after which remission was demonstrated both clinically and on images. Analysis of a frozen section obtained during the procedure aided in the diagnosis of the tumor, thus preventing further resection and the potential neurological complications associated with more radical resection. To the authors’ knowledge this is the first reported case of a presumed primary intradural extraarachnoid Hodgkin lymphoma.

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Christopher R. Honey, Murray D. Morrison, Manraj K. S. Heran and Baljinder S. Dhaliwal

Inducible laryngeal obstruction has been described under at least 40 different monikers, including vocal cord dysfunction, paroxysmal vocal fold motion, and irritable larynx. The etiology of this condition is believed to be laryngeal hyperactivity in response to psychological issues or acid reflux. Most patients are treated with some combination of proton pump inhibitors, speech therapy, and psychotherapy. However, a small cohort of patients remains refractory to all medical interventions. The authors describe a novel condition, hemi-laryngopharyngeal spasm (HELPS), which can cause severe episodic stridor leading to unconsciousness in association with cough. The first recognized and surgically cured patient with HELPS was reported in an earlier issue of this journal. Three additional patients have been followed up for at least a year postoperatively, and their cases are reported here.

Each patient presented with a similar pattern of episodic coughing and choking that increased in frequency, severity, and duration over years. The episodes eventually occurred while sleeping and could cause severe stridor with loss of consciousness. All three patients were initially misdiagnosed with a psychiatric illness and subjected to multiple intubations and one tracheostomy. Unilateral botulinum toxin injections in the vocal fold eased the severity of the throat contractions but not the cough. Magnetic resonance imaging showed a looping posterior inferior cerebellar artery juxtaposed to a vagus nerve in each case. Microvascular decompression (MVD) of that vessel relieved all symptoms.

The introduction of this new medical condition may help a small cohort of patients with inducible laryngeal obstructions that have not responded to the current standard treatments. Patients are asymptomatic between episodes of progressively severe coughing and choking with stridor that may lead to intubation. Severe anxiety about the unpredictable symptoms is expected and may contribute to a psychiatric misdiagnosis. Microvascular decompression for HELPS is more difficult than that for trigeminal neuralgia because the involved nerve is more susceptible to manipulation. Ultimately, the final proof that HELPS is a real and distinct syndrome will require its recognition and successful treatment by colleagues around the world.

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Anthony M. Kaufmann

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C. Michael Honey, Zurab Ivanishvili, Christopher R. Honey and Manraj K. S. Heran


The location of the human spinothalamic tract (STT) in the anterolateral spinal cord has been known for more than a century. The exact nature of the neuronal fiber lamination within the STT, however, remains controversial. After correlating in vivo macrostimulation-induced pain/temperature sensation during percutaneous cervical cordotomy with simultaneous CT imaging of the electrode tip location, the authors present a modern description of the somatotopy of the human cervical STT.


Twenty patients underwent CT-guided percutaneous cervical cordotomy to alleviate contralateral medication-refractory cancer pain. Patient responses to electrical stimulation (0.01–0.1 V, 50 Hz, 1 msec) were recorded and the electrode location for each response was documented with a contemporaneous CT scan. In a post hoc analysis of the data, the location for each patient’s response(s) was measured and drawn on a diagram of their cord. Positive responses were represented only when the lowest possible voltage (≤ 0.02 V) elicited a response. Negative responses were recorded if there was no clinical response at 0.1 V.


Clinically, patients did well with an average reduction in opiates of 75% at 1 week, and 67% were able to leave the palliative care unit. The size of the cervical cord varied between patients, with an average lateral extent (width) of 11 mm and a height of 9 mm. Responses from the lower limb were represented superficially (lateral) and posteriorly within the anterolateral cord. The area with responses from the upper limb was larger and surrounded those with responses from the lower limb primarily anteriorly and medially, but also posteriorly.


In this study, the somatotopic organization of the human STT was elucidated for the first time using in vivo macrostimulation and contemporaneous CT imaging during cordotomy. In this cohort of patients, the STT from the lower-limb region was located superficially and posteriorly in the anterolateral quadrant of the cervical cord, with the STT from the upper-limb region surrounding it primarily anteriorly and medially (deep) but also posteriorly. The authors discuss how the previous methods of cordotomy may have biased the earlier versions of STT lamination. They suggest that an ideal spinal cord entry site for cordotomy of either the upper- or lower-limb pain fibers is halfway between the equator and anterior pole of the cord.